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High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
Published on: January 19, 2022
Reduced anaerobic and aerobic performance in children with primary ciliary dyskinesia
Senem Simsek1, Deniz Inal-Ince2,3, Aslihan Cakmak1
1Faculty of Health Sciences, Department of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Turkey.
Insights
Children with primary ciliary dyskinesia (PCD) show reduced anaerobic and aerobic performance. Age independently predicts anaerobic capacity, while gender determines aerobic performance in these children.
Area of Science:
- Pediatric Pulmonology
- Exercise Physiology
- Rare Diseases
Background:
- Primary ciliary dyskinesia (PCD) is a genetic disorder affecting cilia function, leading to chronic respiratory infections and reduced quality of life.
- Exercise performance, crucial for daily activities and overall health, is often compromised in chronic pediatric conditions.
- Limited research exists on the specific impacts of PCD on both anaerobic and aerobic exercise capacities in children.
Purpose of the Study:
- To investigate and compare anaerobic and aerobic performance in children diagnosed with PCD versus healthy controls.
- To identify factors influencing anaerobic and aerobic capacity in children with PCD.
- To establish the early-stage impact of PCD on physical performance.
Main Methods:
- A cohort of 31 children with PCD and 29 age- and sex-matched healthy controls participated.
- Evaluations included pulmonary function tests, hand grip strength (HGS), quadriceps strength (QMS), and physical activity assessment.
- Anaerobic capacity was measured using a muscle power sprint test, and aerobic performance was assessed via the modified shuttle walk test (MSWT).
Main Results:
- Children with PCD exhibited significantly lower pulmonary function, HGS, QMS, mean anaerobic power (MAP), and MSWT distance compared to healthy subjects.
- In PCD patients, MAP was independently predicted by age.
- Aerobic performance (MSWT distance) in PCD was independently predicted by gender, with males performing better.
Conclusions:
- Both anaerobic and aerobic exercise capacities are significantly impaired in children with PCD from early stages.
- Age is a key determinant of anaerobic performance, while gender influences aerobic performance in PCD.
- Further research is needed to explore the role of skeletal muscle characteristics and sex-based body composition differences in PCD-related exercise capacity deficits.
Abstract:
Primary ciliary dyskinesia (PCD) restricts lifestyle and increases morbidity. The aim of the study was to investigate anaerobic and aerobic performance in children with PCD and their healthy counterparts. Thirty-one children with PCD and 29 age- and sex-matched healthy subjects were studied. Pulmonary function, hand grip strength (HGS), quadriceps strength (QMS), physical activity, anaerobic capacity (muscle power sprint test), and aerobic performance (modified shuttle walk test (MSWT)) were determined. Pulmonary function, HGS, QMS, mean anaerobic power (MAP), and MSWT distance in PCD were significantly lower than those of healthy subjects (p < 0.05). In PCD, the MAP was significantly correlated with age, FEV1, and the mean kcal for 3 days (p < 0.05), and age was its independent predictor (p < 0.05). The MSWT distance was significantly related to gender and weight (p < 0.05), and gender was selected as its independent predictor (p < 0.05). In healthy controls, the MAP was significantly associated with age, gender, FVC, FEV1, HGS, QMS, and the mean kcal for three days (p < 0.05). The MSWT distance was significantly related to weight and body mass index in healthy group (p < 0.05).
Conclusion:
Anaerobic and aerobic performance is impaired in PCD from the early stages. Age determines anaerobic performance. Gender is the determinant of aerobic performance. Whether skeletal muscle characteristics and sex-related changes in body composition affect anaerobic and aerobic capacity in PCD children warrants further study. What is Known: • Exercise performance is determined by anaerobic and aerobic power. • Few studies have shown that PCD patients have lower aerobic performance which is associated with impaired lung function. What is New: • The present research indicated that both anaerobic and aerobic exercise capacity determined using field testing is impaired in PCD from the early stages. • Anaerobic capacity was found to be independently associated with age in PCD. Higher aerobic performance is independently associated with male gender.
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